Patients With Systemic Sclerosis Should Undergo Screen for PAH


The incidence of pulmonary arterial hypertension in patients with systemic sclerosis is 0.61 per 100 patient-years, according to data on 384 patients in a longitudinal study presented at the annual meeting of the European League Against Rheumatism in Paris.

The prevalence of pulmonary arterial hypertension (PAH) in a cohort of patients from the ItinerAir-HTAP registry, which is a 3-year, multicenter study of patients with systemic sclerosis, was found to be 7.85% (confidence interval range, 5.70–10.00), prompting this study to determine the incidence of PAH over 3 years of follow-up, explained Dr. Eric Hachulla of Hôpital Claude Huriez, Lille, France.

The patients underwent Doppler echocardiography screening for PAH. PAH was suspected in those with peak velocity of tricuspid regurgitation (VTR) of 2.8–3 m/sec and unexplained dyspnea, or with VTR greater than 3 m/sec, according to Dr. Hachulla.

Right heart catheterization (RHC) was used to confirm the presence of pulmonary hypertension.

The patients, 87% of whom were women, had a mean age of 53 years and were followed for a mean of 41 months.

Pulmonary hypertension was found in 18 patients (incidence of 1.37 per 100 patient-years).

Of those 18 patients, 8 were found to have pre-capillary pulmonary hypertension identified by RHC, and 8 had post-capillary hypertension detected despite the absence of left heart dysfunction on echocardiography (incidence of 0.61 per 100 patient-years for both groups).

The remaining two patients had pulmonary hypertension resulting from severe interstitial lung disease, Dr. Hachulla noted.

The findings show that post-capillary pulmonary hypertension is common in systemic sclerosis, which indicates that RHC is necessary to confirm pre-capillary PAH, he concluded.

Right heart catheterization is necessary to confirm pre-capillary PAH. DR. HACHULLA

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